Sarcomatoid Mesothelioma: Future Advances in Diagnosis, Biomolecular Assessment, and Therapeutic Options in a Poor-Outcome Disease

Abstract

Malignant pleural mesothelioma (MPM) is the most frequent pleural neoplasm, with asbestos exposure as one of the recognized carcinogen agents, causative in 80% of cases. The prognosis is poor; median survival of untreated cases is 6-9 months, with fewer than 5% of patients surviving 5 years. Sarcomatoid mesothelioma (SM) represents the subtype with the worst outcome and median survival ranging from 3.5 to 8 months. In the last few years, an accurate differentiation between the subtypes of MPM has become a crucial issue, due to differences in chemosensitivity and clinical outcome, and several studies have evaluated different immunohistochemical markers to better define the diagnosis. The different and worse outcome of patients with SM and, in general, nonepithelioid subtypes makes it intriguing to select these cases to better study the biomolecular profile in order to find factors linked to prognosis and/or predictive of therapeutic response. Considering recent studies on miRNA and genetic mapping, further investigation of this rare subtype might represent a field for basic and clinical-translational research providing for more tailored therapies.

Multiple intracranial metastases from postoperative giant sarcomatoid malignant pleural mesothelioma: A case report and literature review

“…Approximately 80% of patients with MPM have a history of asbestos exposure ( 12 ). The period between asbestos exposure and the onset of MPM is reported to be ~30-40 years ( 13 ).…”

“…The period between asbestos exposure and the onset of MPM is reported to be ~30-40 years ( 13 ). MPM originates from the mesothelium of the parietal pleura and is associated with a poor prognosis ( 2 , 12 ), with a median survival period of 11.5–15.3 months. However, SMPM carries a significantly worse prognosis, with a median survival of 4.2–5.0 months ( 14 , 15 ).…”

“…However, SMPM carries a significantly worse prognosis, with a median survival of 4.2–5.0 months ( 14 , 15 ). Only early-stage EPP may prolong the survival of patients with MPM ( 16 ); however, as MPM is either asymptomatic or associated with a non-specific presentation, early diagnosis is usually difficult ( 2 , 12 ).…”

Update on Diagnosing and Reporting Malignant Pleural Mesothelioma

“…Sarcomatoid mesothelioma is less frequent but more aggressive than epithelioid mesothelioma, with mean survivals of 3.5-8 months (12). Differentiating between epithelioid and sarcomatoid MM is important because of stage-dependent differences in treatment approach (13).…”

Precision Therapy for Mesothelioma: Feasibility and New Opportunities

“…However, phenotypically there is a clear gradient of epithelial–mesenchymal transition or EMT [ 5 , 6 , 7 ] which may underpin chemotherapy resistance and the most aggressive behaviour of mesenchymal-like sarcomatoid MPMs. Patients with sarcomatoid MPM tend to have the worst outcomes with median survival ranging between 3.5 to 8 months [ 8 ], considerably shorter than for epithelioid subtype [ 9 ]. To date, although EMT exhibits plasticity, targeting EMT to revert a mesenchymal-to-epithelial phenotype has proven to be challenging [ 10 ].…”